Heidi Shafland, MSN, APRN, ACCNS-P, CCRN-K; Robert Horvath-Csongradi, MD; Francis Moga, MD; Felicia Loney, RD; David Overman, MD; Robroy MacIver, MD; David Dassenko, MD; Jennifer Abuzzahab, MD; Nancy Slater, OTR-L; Mark Lo Galbo
Case Presentation
We had a six-month-old patient with Heterotaxy Syndrome and associated Complex Congenital Heart Disease, including: dextrocardia, malposition of the great vessels, hypoplastic pulmonary valve, tricuspid atresia, bilateral superior vena cavae, right aortic arch. He was admitted for a bilateral cavopulmonary anastomosis. Due to morbidity associated with his illness, he was hospitalized for three months. Midway through his stay, his daily x-ray was significant for healing rib fractures without any associated cause. Laboratory values showed significant hypovitaminosis D. Supplementation was started and the plan of care was changed to gentle handling. A week later, the nurse noticed he appeared to be in significant pain with movement. A femur x-ray demonstrated a healing proximal femur fracture. After ruling out all other causes for bone weakness, he was diagnosed with osteopenia. A multidisciplinary group at Children’s Minnesota Cardiovascular Intensive Care Unit convened to discuss how to identify and treat this fragile patient population.
Definitions
Once considered an “old person’s disease,” threats to bone health are increasingly common due to genetic illnesses affecting the bone, chronic illnesses and their medical treatments. Patients with osteopenia are at risk for inappropriate absorption and decreased bioavailability of essential electrolytes for bone growth, decreased bone mineral density, and if severe, fractures.
Osteopenia is defined as a decrease in the amount of organic bone matrix. There are not sufficient amounts of elemental materials to create strong bones.
Osteomalacia is defined as a lack of mineralization of the organic bone matrix. A sufficient amount of elemental materials to create bones exists, but it is not effective in creating strong bones.
Rickets is when the lack of mineralization involves the growth plate, stunting a child’s growth.
Osteoporosis is a decrease in bone mineral density >2.5 SD from the norm. Unfortunately, the norm is poorly defined for pediatrics. The reason why a definition of “normal” has not been possible is the rates of bone growth and mineral accrual are more closely linked to pubertal and skeletal maturation than to chronological age. The DEXA scan, which is the gold standard in diagnosing osteoporosis in adults, only provides a two-dimensional measurement of the three-dimensional skeleton. Several methods have been developed to account for bone size and create a “normal,” for pediatrics; none of them have been effective.
Lastly, metabolic bone disease is the preferred term for osteopenia in pediatrics.
Project Goals
Identify risk factors which make patients more susceptible to osteopenia
Follow laboratory values to monitor these patients
Physical and occupational therapy for patients identified as high risk for osteopenia
Prevent future bone fractures
Introduction
The body of literature that describes osteopenia in the pediatric cardiac population is based primarily on expert opinions and a few case studies. What we learned was extrapolated from adult or premature infant literature. Cheng et. al assessed fractures in pediatric patients with Congenital Heart Disease and found patients with osteopenia had an increased mortality rate; hyperparathyroidism was present in the majority of their patients and low Vitamin D was present in almost half their population.[1] They found the creation of a protocol to identify and treat patients resulted in decreased numbers of fractures. Cross et. al discussed nursing implications for osteopenia in the premature patient, supporting the difference nursing interventions can make.[2] This study addressed nursing interventions to guide handling patients with osteopenia. Marrani et. al has a well-defined description of osteopenia: “all patients are ‘at risk’ of osteopenia until they have a fracture, when a definitive diagnosis can be made.”[3]. The true incidence of pediatric osteopenia is difficult to assess, because there are very few effective methods of assessing bone demineralization. Bachrach et. al described what a comprehensive bone assessment should include in their study.[4] Khosla et. al discussed the risks and benefits of bisphosphonate use in children, which is an accepted treatment for osteoporosis in adults. Bisphosphonates lower fracture risk in large part by reducing the rate of bone remodeling as well as by increasing bone mass. Treatment with this medication class is not without risks, however. Some of the risks of bisphosphonate use include: electrolyte imbalances, renal toxicity, atrial fibrillation, osteonecrosis of the jaw, and subtrochanteric fractures.[5] Umass et. al explained how different chronic pediatric diseases contribute to the development of secondary osteoporosis.[6]
To read the full article, please go to the August 2019 Issue of CCT.